Nasir Saeed, Salim Khan, Muhammad Daud.
Death associated with Acute Posterior Multifocal Placoid Pigment Epitheliopathy.
Pak J Ophthalmol Jan ;19(1):22-4.

We report a case where a patient died suddenly one week after diagnosis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Her systemic examination and Laboratory investigations including CT brain were unremarkable. APMPPE has previously been reported as a cause of sudden death in two patients. It is important to have a high index of suspicion for unusual symptoms or signs in APMPPE. Recognition of life-threatening complications of APMPPE may permit early treatment and prevention of morbidity and mortality. informing patients regarding symptoms of potential neurological complications is highly recommended.

Case Report: A 35-year-old woman presented with sudden visual blurring in one eye followed by the second eye for ten days and occasional mild headaches. Her initial visual acuity was counting fingers at l meter in each eye. The anterior chamber showed inflammatory aqueous cells. Ophthalmoscopy revealed multiple yellow-white sub-retinal patches at the posterior pole and slight blurring of the nasal optic disc margins in both eyes. Fundus fluorescein angiography showed early hypofluorescence of the lesions followed by the late hyperfluorescence. Systemic examination was unremarkable. Her complaint of headaches prompted a computed tomography (CT) of the brain, which was normal. Routine investigations including chest roentgenography were unremarkable. The serum anti-nuclear antibody test was negative and serum angiotensin converting enzyme level was within normal limits. She was diagnosed as a case of APMPPE and was prescribed topical dexamethasone and oral prednisone (60 mg/day). A few days later her visual acuity had improved to 6/24 in each eye. Anterior segment inflammation also subsided. She was discharged from hospital and on her way home, she experienced severe headache, vomiting and drowsiness. She was taken to another hospital where she reached in a state of coma. She was put on life support treatment but died later the same day.

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